Nasal Construction in Congenital Arhinia Due to Novel SMCHD1 Gene Variant.

Arhinia, or congenital absence of the nose, is an exceedingly rare anomaly caused by pathogenic variants in the gene SMCHD1 . Arhinia exhibits unique reconstructive challenges, as the midface is deficient in skeletal and soft tissue structures. The authors present 2 related patients with arhinia who harbor a novel SMCHD1 gene variant and illustrate their surgical midface and nasal construction. Targeted sequencing was carried out on DNA samples from the 2 affected patients, 1 anosmic and 1 healthy parent, to identify variants in exons 3 to 13 of SMCHD1 . The affected patients and anosmic parent were found to have a novel SMCHD1 gene variant p.E473V. A staged surgical approach was applied. First, both patients underwent a LeFort II osteotomy and distraction osteogenesis to improve the projection of the midfacial segment, followed by tissue expansion of the forehead, and nasal construction with a forehead flap that was placed over a costochondral framework derived from rib cartilage. The novel gene variant could guide future investigations on genetic pathways and molecular processes that underly the physiological and pathologic development of the nose. Further investigations on the variable expressivity ranging from anosmia to arhinia could improve clinical genetic screens for risk stratification of individuals with anosmia on passing on arhinia to their children. Due to the exceptional rarity and complexity of congenital arhinia, most surgical approaches are developed on a single-case basis. This case series, albeit limited to 2 cases, is the largest pedigree of such cases in the literature. It highlights key principles of a staged approach to nasal construction in arhinia and discusses nuances and improvements learned between both patients. It subsequently offers an optimized guide to this surgical strategy.

Pediatric Postburn Ear Reconstruction of Significant Cartilage Defects.

The external ear is a vulnerable appendage susceptible to trauma. Repairing and recreating its intricate architecture presents a formidable challenge after burn injury, especially when scar tissue, impaired vasculature, and damage to cartilage all influence reconstructive options. The authors share their institutional experience and provide a guide for reconstruction of significant postburn auricular cartilage defects. A retrospective review was conducted on 54 patients (61 ears) aged 0-21 years old admitted to a specialized pediatric burn hospital between January 2004 and January 2021 for postburn ear cartilage reconstruction. Conchal cartilage grafting was performed in 9 patients (9 ears) with an average age of 14.0 ± 5.1 years, requiring an average of 2.0 ± 1.0 procedures that resulted in one case of infection. A total of 21 patients (24 ears) with an average age of 15.1 ± 4.5 years underwent a conchal transposition flap, requiring an average of 1.8 ± 0.4 procedures that resulted in no major complications. A total of 20 patients (23 ears) with an average age of 12.0 ± 5.3 years underwent porous polyethylene implantation, requiring an average of 3.5 ± 1.5 operations that resulted in 3 complications. Costal cartilage grafting was performed in 4 patients (5 ears) with an average age of 13.2 ± 5.3 years, requiring an average of 3.2 ± 2.2 operations that resulted in one case of infection. Postburn ear cartilage defects necessitate an individualized approach tailored to a patient's reconstructive goals. In addition to defect size and location, factors such as reconstructive course duration, complication potential, and anticipated aesthetic results should be discussed with the patient.

Topical therapy for regression and melanoma prevention of congenital giant nevi.

Giant congenital melanocytic nevi are NRAS-driven proliferations that may cover up to 80% of the body surface. Their most dangerous consequence is progression to melanoma. This risk often triggers preemptive extensive surgical excisions in childhood, producing severe lifelong challenges. We have presented preclinical models, including multiple genetically engineered mice and xenografted human lesions, which enabled testing locally applied pharmacologic agents to avoid surgery. The murine models permitted the identification of proliferative versus senescent nevus phases and treatments targeting both. These nevi recapitulated the histologic and molecular features of human giant congenital nevi, including the risk of melanoma transformation. Cutaneously delivered MEK, PI3K, and c-KIT inhibitors or proinflammatory squaric acid dibutylester (SADBE) achieved major regressions. SADBE triggered innate immunity that ablated detectable nevocytes, fully prevented melanoma, and regressed human giant nevus xenografts. These findings reveal nevus mechanistic vulnerabilities and suggest opportunities for topical interventions that may alter the therapeutic options for children with congenital giant nevi.

Inflammatory and infectious complications of laser therapy in treatment of hypertrophic burn scars: Correlations in literature review & case reports.

INTRODUCTION: Hypertrophic burn scars contribute to morbidity through secondary symptoms of pain, pruritus, and scar contracture. Traditional treatment methods are now augmented by the use of monochromatic light therapies, which are generally accepted as safe and effective. However, little literature is available regarding the complications of laser treatments of hypertrophic burn scars and even less regarding inflammatory and infectious complications. METHODS: A literature search using PubMed was performed to identify literature pertaining to infectious and inflammatory complications of cutaneous laser treatments. Additionally, we reviewed cases of inflammatory and infectious complications occurring at our institution after laser treatment of hypertrophic burn scars. RESULTS: We identified 1 publication related to complications of laser therapy in the treatment of burn scars. In this series of 163 laser sessions, the reported incidence of adverse events was 25.1%, of which 6 cases 3.7% were related to inflammatory and infectious processes. In the 391 laser sessions performed at our institution (December, 2015 and July, 2016) 9 cases of inflammatory and infectious complications were noted yielding an incidence of 2.3%. Cases included 3 each of cellulitis, Systemic Inflammatory Response Syndrome (SIRS), and complicated SIRS. CONCLUSION: We found the most common inflammatory complication was SIRS with MSSA positive wound cultures. Three cases underwent hospitalization along with fluids and vasopressors, despite negative blood cultures. In light of the high prevalence of MSSA in the natural skin flora and negative blood cultures, the inability to establish a true source of infection lead to declaring these cases "complicated SIRS" and not sepsis. Correlative factors that may have led to complications reported in our cases were: preoperative evidence of infection, no preoperative antibiotics administered, no postoperative antibiotic dressings, combined procedures, and large treatment areas. The true mechanism of inflammatory and infectious complication is yet to be determined, but we postulate that these factors place a greater challenge on an already burdened immune system. Determining whether this is a true causal mechanism, leading to an aggravated inflammatory response, benefits from further investigation. APPLICABILITY OF RESEARCH TO PRACTICE: We urge institutions preforming such procedures to advise patients on preoperative wound preparation. We recommend that each individual with a preexisting history of infection and/or preoperative culture evidence of infection receive antibiotics, particularly when undergoing combined procedures or procedures involving higher surface areas. Although complications are rare, the benefits of these precautionary measures outweigh the risks when it comes to prevention and management.

Evaluation of liposomal bupivacaine infiltration at reconstructive skin graft donor sites in adolescent and young adult burn patients: A retrospective analysis.

BACKGROUND: Postoperative pain at skin graft donor sites is frequently undertreated in burn patients, which can impair reconstructive outcomes and result in harmful psychological consequences. We find a critical need to explore and promote non-opioid, multimodal analgesics. Donor site infiltration of the local anesthetic liposomal bupivacaine in adolescent and young adult burn patients has not been previously investigated. Therefore, the goal of this study was to evaluate intraoperative liposomal bupivacaine infiltration for postoperative donor site pain control in adolescent and young adult burn patients undergoing reconstructive skin graft procedures. METHODS: This retrospective analysis included patients aged 14-25 years, who underwent at least two reconstructive skin graft procedures, one that received donor site infiltration of the standard treatment (bupivacaine hydrochloride) and one that received donor site infiltration of liposomal bupivacaine. The final sample included 30 patients with a total of 44 liposomal bupivacaine cases and 53 standard treatment cases analyzed. RESULTS: In the authors' five-year experience, the use of liposomal bupivacaine compared to standard treatment was associated with statistically significant decreases in 0-4 h postoperative pain scores (mean 1.4/10 versus 2.3/10, p = 0.04) and 0-24 h postoperative pain scores (mean 1.7/10 versus 2.4/10, p = 0.02). Neither analgesic was associated with adverse events. Differences in length of stay and inpatient postoperative opioid usage were not regarded as significant. CONCLUSION: In this retrospective analysis, the authors report the first results that suggest intraoperative liposomal bupivacaine donor site infiltration may be associated with statistically improved patient outcomes in adolescent and young adult burn patients. However, the reported differences are most likely not clinically significant, establishing the necessity for further evaluation of using liposomal bupivacaine in this unique patient population.

Experience of outreach in a resource-constrained environment: 10 years of outcomes in burn care.

OBJECTIVE: The objective of this work was to describe an efficient and sustainable outreach model in a resource-constrained environment, with a multifaceted approach focusing on national policy change, telemedicine, injury prevention, education and treatment of burns. SUMMARY BACKGROUND DATA: Burn injury constitutes a significant portion of morbidity and mortality worldwide, particularly in children, and in low- and middle-income countries. METHODS: We reviewed the impact of ten-years of a burn outreach program. Our focus was on clinical data on burn care within one region of Ukraine. We assessed knowledge of burn prevention/first aid utilizing a large survey, analyzed clinical data from our outreach clinic and telemedicine program, and analyzed data within a newly created burn repository within Ukraine. RESULTS: A national burn prevention policy has been implemented through our efforts along with a burn prevention program. Educational efforts have led to improvements of major complication rates (wound infection [7% vs. 16%], pneumonia [2.4% vs. 0.3%], sepsis [1.6% vs. 0.6%], UTI [2% vs. 0.6%], and cellulitis [11% vs. 3.4%]), respiratory support of acutely ill patients [1.3% vs. 0.4%], and blood transfusion triggers. CONCLUSIONS: Broadly, our model could be an example of building sustainable outreach programs in resource-constrained environments. Through collaboration with local healthcare providers, we have developed and implemented an outreach program in a resource-constrained environment.

Antegrade Foreheadplasty: A Novel Surgical Approach for Upper Eyelid Contracture Deformities in Pediatric Patients.

The intricate and delicate structure of the periorbital region, particularly in pediatric patients, presents challenges to eyelid reconstruction. Much like the more common lower eyelid ectropion, upper eyelid ectropion can result from lack of tissue, scar contracture, or over-resection as in blepharoplasty. In burns and trauma, the cause of cicatricial ectropion is typically direct scar contracture from injuries to the eyelid. However, in some cases, extrinsic wounds involving contracture to the forehead or eyebrow can result in upper eyelid cicatricial ectropion. Direct reconstruction and skin grafting of the eyelid present complex challenges, especially in the acute inflammatory phase of traumatic injury and burn care. Furthermore, in many of these cases the periorbital and lamellae anatomy is preserved, but rather severely displaced due to scar contracture forces. The authors discuss our experience with treatment of extrinsic upper eyelid cicatricial ectropion in a series of 4 pediatric patients with burns or trauma to the forehead and periorbital regions. In all 4 cases, the antegrade foreheadplasty procedure helped to provide globe coverage, while avoiding skin matching difficulties and the intrinsic risks of operating on the eyelid during the acute phase of recovery. There is currently very limited data for the use of this technique to correct such defects. With this study, the authors hope to establish the antegrade foreheadplasty as a reconstructive option for a select patient population.

Latissimus dorsi free flap phalloplasty: a systematic review.

A phalloplasty is a complex genital reconstruction procedure of creating a neophallus. Several techniques to create a neophallus are described, based on different vascularized flaps, and each of them has its advantages and drawbacks. The aim of this study is to present musculocutaneous latissimus dorsi (MLD) flap as a viable option for total phalloplasty, with an interest in clinical outcomes and complications. A comprehensive literature review of all available reports about MLD flap phalloplasty was made. The following keywords were used on PubMed: latissimus dorsi musculocutaneous/myocutaneous free flap and phalloplasty. Research criteria revealed five articles and the results of 182 patients were analyzed. A total number of the patients, indications, operative technique, follow-up period, postoperative results, and complications were presented. In conclusion, MLD free flap presents a good choice for phalloplasty providing sufficient amount of tissue for safe implantation of penile prosthesis and successful penetrative sexual intercourse. The erogenous sensitivity is preserved with clitoris or glans penis incorporated into the base of the neophallus, and voiding in a standing position is achievable after urethral reconstruction. The main drawback is the lack of tactile sensation of the neophallus and the significant advantage is a well-concealed donor site.

Triangular Fossa Graft for Philtral Reconstruction After Facial Burns: A 34-Year Experience.

BACKGROUND: Upper lip burns and skin grafts used for the resulting deformities all contract, leading to distortion of regional tissues and producing a flattened upper lip devoid of normal anatomic landmarks. Absence of the philtral contour draws attention to the upper lip and can accentuate other reconstructive inadequacies. Philtral restoration requires a 3-dimensional reconstruction capable of resisting contractile forces to restore and maintain normal relationships between the upper and lower lips. METHODS: This was a 34-year retrospective review of a single surgeon's experience using a composite triangular fossa graft from the ear for philtral reconstruction. Ten patients were identified and analyzed using records of follow-up examinations, long-term clinical evaluations, and photographic documentation. RESULTS: Five males and 5 females were identified with 2- to 34-year follow-up. Age at operation ranged from 14 to 52 years. Percent total body surface area ranged from less than 1% to greater than 90%. Previous upper lip grafts prior to the auricular graft included 5 full-thickness skin grafts and 5 split-thickness skin grafts. No significant complications were noted. All patients were satisfied with the end aesthetic result and donor site morbidity. CONCLUSIONS: A triangular fossa composite graft restores and preserves the philtral dimple and corrects the obvious visible deformity of a featureless upper lip. Addition of tissue loosens the lip transversely, and the cartilage component provides a consistent and predictable upper lip position. This results in improved projection, a concave shape to the upper lip, and a better relationship with the lower lip. Restoration of this anatomic landmark creates a more normal-appearing upper lip and helps to minimize the negative impact of other abnormalities in this vitally important area. The overall improvement in total facial appearance can be profound.

Fractional Laser-assisted Hair Regrowth and Microneedling for the Treatment of Alopecia Areata: A Review.

Alopecia areata (AA) affects approximately 2.1% of the population, with women being affected more often than men. Current therapies consisting of topical corticosteroids or intralesional injections are often the first choices for treatment, but are limited by unsatisfactory outcomes or risks to patients. Recently, fractional lasers and microneedling, with or without the addition of topical agents, have been examined as treatment options. A literature review was performed to evaluate the efficacy of fractional lasers in the treatment of AA. A total of six fractional lasers and two microneedling studies consisting of small prospective and retrospective studies, and case reports were reviewed. The number of trials and participants are limited, but evidence suggests that fractional lasers and microneedling may be effective therapeutic approaches when coupled with topical agents. Larger studies are required to better understand the effects of these treatment modalities for AA.

Laser-assisted Hair Regrowth: Fractional Laser Modalities for the Treatment of Androgenic Alopecia.

BACKGROUND: A large proportion of the population is at sometime affected by androgenic alopecia. Current therapies consisting of minoxidil or finasteride are often the first choices for treatment. These regimens are limited by their efficacy, side-effect profiles, and often lengthy treatment courses. Low level laser/light has shown to be relatively effective and safe for the treatment of hair loss, and a number of products are currently available to consumers. Recently, fractional lasers have been examined as treatment options for androgenic alopecia. The mechanism of action of these minimally invasive resurfacing procedures is thought to be 2-fold. First, the microscopic injuries created by these treatments may induce a favorable wound healing environment that triggers hair growth. Alternatively, disruption of the stratum corneum allows for improved transdermal passage of well-established therapeutic drugs to the hair roots. METHODS: A literature review was performed to evaluate the efficacy of these emerging treatments on hair regrowth. RESULTS: Nine original studies examining the effect of fractional lasers on hair growth in androgenic alopecia have been reviewed. CONCLUSIONS: Preliminary evidence suggests that fractional laser therapies have a positive effect on hair regrowth; however, most of the literature is limited to case reports, and small prospective and retrospective series. Further studies, in the form of well-designed randomized controlled trials, are necessary to evaluate the efficacy, safety, and optimal treatment courses.

Burn Depth Analysis Using Indocyanine Green Fluorescence: A Review.

Distinguishing between partial-thickness burns and full-thickness burns indicates the critical decision making related to whether a patient can be allowed to heal on their own or if the wound should be surgically excised and replaced with a graft. Therefore, reliable burn prognostication is crucial in planning the safest and most cost-effective treatment for the patient. Over the course of the last several decades, many techniques have been proposed and used to determine burn depth; namely, laser Doppler imaging and thermogenic assessment. Recently, indocyanine green (ICG), a fluorescent dye which is administered intravenously to measure tissue perfusion in real time, has been the focus of research regarding its efficacy in assessing burns. Studies have been conducted using rat, porcine, and human models. We have assessed and critically reviewed this literature to provide the burn community with a narrative of the promising potential of ICG to diagnose burn depth. ICG can measure perfusion instantaneously and in real time via video capture. This allows for evaluation of ICG uptake, standard state distribution, and clearance of the dye which with further study could increase the precision of the technique. In conclusion, ICG videoangiography shows a great deal of promise in being a particularly effective way of diagnosing burn depth and warrants further studies to investigate the best way for this technique to be used in a clinical setting.

Short Runs for a Long Slide: Principalization in Complex Facial Restoration after Acid Attack Burn Injury.

Burn injuries are responsible for a significant portion of surgically treatable morbidity throughout the world and particularly in underdeveloped and developing countries. Intentional flame, chemical, and contact burns are unfortunately a common mechanism of injury. It is estimated that intentional chemical burns are responsible for between 2 and 20% of burn injuries seen at burn centers in lower income countries. Women are commonly targeted and the perpetrators are often known to the victims. The combination of a high disease prevalence, limited surgical and anesthetic resources, a vulnerable patient population, and largely disfiguring, nonlethal injuries present unique challenges for the reconstructive surgeon who may not encounter such cases regularly. In this article, we present a case of a 16-year-old female who sustained severe, full-thickness burns to the face including eyelids, neck, abdomen, and upper extremities after an intentional acid attack. She began her treatment course with us approximately 1 year after the injury. The deformities of her oral and periorbital regions presented particularly difficult reconstructive problems, including impending visual loss. Using plastic surgical principalization, we provided our patient adequate restoration of facial form and function through numbers of interventions using fundamental and state-of-the-art techniques.

Reconstruction of Lumbar Spinal Defects: Case Series, Literature Review, and Treatment Algorithm.

BACKGROUND: Lumbar spinal defects present a distinct challenge for the reconstructive surgeon and are often complicated by previous spinal surgery, the presence of hardware, and prior radiation. There are a variety of reconstructive options described but no clear treatment algorithm for these challenging defects. METHODS: A literature search was performed to review the described treatment options for lumbar spinal soft-tissue defects. Treatments for myelomeningocele or pressure ulcers were excluded. In addition, a retrospective case review was performed for patients with lumbar defects treated with pedicled superior gluteal artery perforator (SGAP) flap reconstruction. RESULTS: A literature review revealed 2,022 articles pertaining to lumbar defect reconstruction, of which 56 met inclusion criteria. These articles described 13 individual reconstructive techniques for this anatomic area. The most commonly described techniques were evaluated and a treatment algorithm designed based on patient and wound characteristics. The clinical course of 5 patients with radiated lumbar spinal defects treated with SGAP flaps is presented. CONCLUSIONS: There are many treatment options for lumbar spinal defects and treatment should be tailored to the patient and the characteristics of the defect. For large or radiated lumbar wounds, the pedicled SGAP flap is an excellent reconstructive choice, as it has reliable anatomy that is outside the zone of injury, adequate bulk, and minimal donor-site deficits.

Epidural Anesthesia to Facilitate Organ Blood Flow During the First Penile Transplantation in the United States: A Case Report.

Regional anesthesia has been used to help create local sympathectomy and improve blood flow in plastic surgery procedures involving tissue grafts and flaps. However, anesthetic techniques that reduce systemic vascular resistance must be used with caution in patients with aortic stenosis (AS). Combined neuraxial and general anesthesia with careful titration of the local anesthetic dose can be a safe approach for patients with AS undergoing microvascular procedures. We present the anesthetic management of the first North American penile transplant, on an obese patient with moderate AS.

Development of a primary melanoma in situ within a full-thickness skin graft overlying a free muscle flap: a case report.

The development of a primary melanoma within the confines of free tissue transfer is a rare occurrence. In this report, we describe the development of a primary melanoma in situ within a full-thickness skin graft overlying a free latissimus dorsi muscle flap used to cover a scalp defect.